Thrombotic Thrombocytopenic Purpura: Diagnosis and Treatment 2 2 Thrombotic Thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathic disease, associated with thrombocytopenia and hemolytic anemia. It is caused by an enzymatic dysfunction responsible in cleavage of blood clotting factors. In this study we have tried to review the available approaches in diagnoses of the disease as well as treatment strategies. Based on the what the current review has provided, treatment policy depends on accurate diagnosis, it seems that developing a diagnostic and treatment guide for this disease is currently essential. Also, it’s necessary to study genetic types of TTP with more details because, hopefully, new techniques of gene therapy open a window to more stable treatments. 111 120 2020/05/27 1399/3/7 2020/10/7 1399/7/16 Mohammadreza Saeidnia Mohammadreza Saeidnia Department of Hematology, School of Paramedical, Shiraz University of Medical Sciences, Shiraz, Iran m.rezasaeednia@gmail.com Faride Nam Avar Jahromi Faride Nam Avar Jahromi Department of Hematology, School of Paramedical, Shiraz University of Medical Sciences, Shiraz, Iran Faridehnamavar96@gmail.com Elnaz Vaziee Elnaz Vaziee Department of Hematology, School of Paramedical, Shiraz University of Medical Sciences, Shiraz, Iran elivaziee@yhoo.com Hossein Dehghani Hossein Dehghani Department of Hematology, School of Paramedical, Shiraz University of Medical Sciences, Shiraz, Iran Dr.hosseind@gmail.com Amir Mahmoodzade Amir Mahmoodzade Medical Biology Research Center, Health Technology Institute, Kermanshah University of Medical Sciences, Kermanshah, Iran Amir.biochem@gmail.com Gholamhossein Tamaddon Gholamhossein Tamaddon Diagnostic Laboratory Sciences and Technology Research Center, School of Paramedical Sciences, Shiraz University of Medical Sciences, Shiraz, Iran tamaddon.g@gmail.com Thrombotic thrombocytopenic purpura Diagnosis Gene therapy ## ##

Investigation of Paternal RhD Zygosity by Two Molecular Methods among Blood Donors in Kurdistan Province, Iran 2 2 Background: RhD antigen system is the leading cause of hemolytic disease of the fetus and newborn (HDFN). Paternal molecular RhD zygosity test is valuable to decide on the use of anti-D immunoglobulin prophylaxis in Rh D-negative pregnant women. We aimed to investigate the paternal RhD zygosity by two molecular methods among blood donors in Kurdistan province, the west of Iran. We also compared these two methods in determining RhD zygosity. Methods: 100 RhD positive blood samples were collected from male blood donors with RhD negative spouses who were referred to Kurdistan Blood Transfusion Center. The phenotype of all samples was tested for Rh D, C, c, E and e antigens by standard hemagglutination methods. Then, RhD zygosity of all samples was evaluated in terms of Rhesus box marker by SSP-PCR and PCR-RFLP methods. Results: Among 100 RhD positive samples, 37% were heterozygote and 63% were homozygote for RhD gene. Both SSP-PCR and PCR-RFLP methods were able to detect zygosity with similar accuracy. Moreover, Rh phenotyping revealed that DCCee (38%) and Dccee (2%) were the most and the least frequent phenotypes in our sample, respectively. Conclusion: RhD zygosity determination in men who have an RhD negative partner by molecular methods such as PCR-SSP and PCR-RFLP could be the first step in preventing HDFN and avoiding unnecessary administration of Rh immunoglobulin in Iran. 121 125 2020/05/272020/02/29 1398/12/10 2020/10/72020/11/2 1399/8/12 Saba Namjoo Saba Namjoo Blood Transfusion Research center, High institute for education and research in Transfusion Medicine, Tehran, Iran sanamjoo18@gmail.com Arezzo Oodi Arezzo Oodi Blood Transfusion Research center, High institute for education and research in Transfusion Medicine, Tehran, Iran ar.oody@gmail.com Naser Amirizadeh Naser Amirizadeh Blood Transfusion Research center, High institute for education and research in Transfusion Medicine, Tehran, Iran amiri_na@yahoo.com Behzad Nazel Khosroshahi Behzad Nazel Khosroshahi Blood Transfusion Research center, High institute for education and research in Transfusion Medicine, Tehran, Iran behzad.n.kh@gmail.com Shirin Ferdowsi Shirin Ferdowsi Blood Transfusion Research center, High institute for education and research in Transfusion Medicine, Tehran, Iran sh.ferdowsi@tmi.ac.ir RhD zygosity Paternal testing Genotyping Molecular tests Hemolytic disease Fetus Newborn Alloimmunization ## ##

Evaluation of Normal Range of Bleeding Scores in Healthy Iranian Adults using the International Society on Thrombosis and Hemostasis Bleeding Assessment Tool 2 2 Background: Bleeding assessment tools are key components in the evaluation of patients suspicious for bleeding disorders. The exact determination of the normal ranges of ISTH-BAT (International Society on Thrombosis and Hemostasis –Bleeding Assessment Tool) in the healthy population is a crucial step for determining who needs to be referred for further coagulation laboratory examinations. We aimed to determine the normal range of ISTH-BAT and simultaneously evaluate the Von Willebrand disease (VWD) diagnostic panel tests in a healthy Iranian group. Methods: ISTH-BAT as well as prothrombin time, activated partial thromboplastin time, factor VIII clotting assay, von Willebrand factor (VWF) antigen, VWF ristocetin cofactor assay, ristocetin induced platelet agglutination, and blood group typing were assessed for 25 normal adults without any bleeding symptoms or a known coagulation disorder. Results: In the 25 studied subjects (13 men, 12 women), the range of bleeding score was 0-6 in women and 0-5 in men; however, since the scores were lower than 2 in most (68%) cases, the interquartile range (IQR) was used for normalizing the data. The ISTH-BAT normal range was found to be 1-4 in women and 0–2 in men. Conclusion: According to this result, the ISTH-BAT cut-off for abnormal bleeding score was ≥5 in adult women and ≥3 in adult men. These data may be valuable in the routine practice of clinicians and adult hematologists in our country for the assessment of individuals with suspected bleeding disorders. 126 130 2020/05/272020/02/292020/04/7 1399/1/19 2020/10/72020/11/22020/12/8 1399/9/18 Omid Seidizadeh Omid Seidizadeh Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Fondazione Luigi Villa, Milan, Italy omidseidi93@gmail.com Minoo Ahmadinejad Minoo Ahmadinejad Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Tehran, Iran minooam@gmail.com Negar Rezaei Negar Rezaei Non-Communicable Diseases Research Center, Endocrinology and Metabolism Research Institute, Tehran University of Medical Sciences, Tehran, Iran n.rezaei81@yahoo.com Bleeding score ISTH-BAT Normal range von Willebrand disease von Willebrand factor Vicenza bleeding questionnaire ## ##

Sub-acute Exposure to Benzene Accelerates the Aging Process of Red Blood Cells; an In vivo Study 2 2 Background: The well-known toxic effects of benzene toxicity are bone marrow depression, reduction in blood cell counts, and induction of leukemia and aplastic anemia. This study was designed to evaluate biomarkers of aging in red blood cells (RBCs). Methods: Mice were exposed to benzene (50, 100, and 200 mg/kg/day) orally for 28 days. A group of benzene-exposed mice were injected intraperitoneally with N-acetylcysteine (NAC, 150 mg/kg/day). Hematological factors, erythrocyte morphology, and sialic acid content of RBCs along with oxidative stress biomarkers were investigated. Results: Benzene dose-dependently reduced RBCs count, hemoglobin level, RBCs membrane sialic acid levels, the total antioxidant capacity of plasma, and G6PD activity of RBCs. The activity of antioxidant enzymes and lactate dehydrogenase, oxidative damage end-products and bilirubin levels, reticulocyte count, and RDW and MCV ranges increased in a dose-dependent manner. Poikilocytosis (spherocyte, burr cell, schistocyte and blister cell) and anisocytosis were observed in high doses of benzene. Conclusion: Our results support the acceleration of RBCs aging and hemolytic anemia in mice exposed to benzene. Co-administration of NAC as an antioxidant effectively alleviated hematotoxicity of benzene. 131 137 2020/05/272020/02/292020/04/72020/04/24 1399/2/5 2020/10/72020/11/22020/12/82020/11/2 1399/8/12 Mokarameh Pudineh Moarref Mokarameh Pudineh Moarref Department of Toxicology and Pharmacology, Pharmaceutics Research Center, Institute of Neuropharmacology, Kerman University of Medical Sciences, Kerman, Iran pudineh.m@gmail.com Moeinadin Safavi Moeinadin Safavi Department of Pathology, Afzalipour Medical Faculty, Kerman University of Medical Sciences, Kerman, Iran safavi_moeinadin@yahoo.com Mohammad-Amin Mostafavi Mohammad-Amin Mostafavi Department of Toxicology and Pharmacology, Pharmaceutics Research Center, Institute of Neuropharmacology, Kerman University of Medical Sciences, Kerman, Iran mohammadaminmostafavi@gmail.com Somayyeh Karami-Mohajeri Somayyeh Karami-Mohajeri Department of Toxicology and Pharmacology, Pharmaceutics Research Center, Institute of Neuropharmacology, Kerman University of Medical Sciences, Kerman, Iran Somayyehkarami@gmail.com erythrocyte senescence oxidative stress sialic acid sub-acute toxicity ## ##

Synchronous Gastrointestinal Stromal Tumor and Axillary Deep Fibromatosis: A Rare Association 2 2 Gastrointestinal Stromal Tumor (GIST) is the most common mesenchymal tumor of the gastrointestinal tract and most commonly affects the stomach, while fibromatosis is a rare locally aggressive fibrous tissue neoplasm. There have been reports of GIST and fibromatosis occurring in same individual and in most of them fibromatosis occurs within the abdomen. In 75% of patients fibromatosis occurs after the diagnosis of GIST while in 20% it is seen synchronously. Here we report a case of axillary fibromatosis with synchronous GIST of the gastroesophageal junction in a 45-year-old male treated with surgery and now on adjuvant imatinib. 138 140 2020/05/272020/02/292020/04/72020/04/242020/02/27 1398/12/8 2020/10/72020/11/22020/12/82020/11/22020/09/22 1399/7/1 Sugeeth MT Sugeeth MT Department of Medical Oncology, Regional Cancer Centre, Trivandrum, India sugeethmt@ymail.com Geetha Narayanan Geetha Narayanan Department of Medical Oncology, Regional Cancer Centre, Trivandrum, India geenarayanan@yahoo.com Gastrointestinal stromal tumor Fibromatosis Gastroesophageal junction ## ##

Sirolimus: A Rapid Treatment for Coagulopathy Because of Vascular Malformations 2 141 142 2020/05/272020/02/292020/04/72020/04/242020/02/272020/02/29 1398/12/10 2020/10/72020/11/22020/12/82020/11/22020/09/222020/09/26 1399/7/5 hossein karami hossein karami Thalassemia Research Center, Faculty of Medicine, Mazandaran University of Medical Sciences, Sari, IR Iran karami_oncologist@yahoo.com Maryam Ghasemi Maryam Ghasemi immunogenetics research center,faculty of medicine,mazandaran university of medical sciences,sari,Iran a.zghasemi@yahoo.com Seyed Abdollah Mousavi Seyed Abdollah Mousavi Department of Pediatric Surgery, Pediatric Infectious Diseases Research Center, Mazandaran University of Medical Sciences, Sari, Iran dr.a.mosavi@gmail.com Mohammad Naderisorki Mohammad Naderisorki Thalassemia Research Center, Faculty of Medicine, Mazandaran University of Medical Sciences, Sari, IR Iran dr.naderisorki@gmail.com ## ##

Factor 13 Activity in Children with Henoch-Schonlein Purpura in Iranian Children 2 143 144 2020/05/272020/02/292020/04/72020/04/242020/02/272020/02/292020/03/20 1399/1/1 2020/10/72020/11/22020/12/82020/11/22020/09/222020/09/262020/10/7 1399/7/16 Vadood Javadi Parvaneh Vadood Javadi Parvaneh Pediatric Pathology Research Center of Shahid Beheshti University of Medical Sciences, Tehran, Iran vadoodj@gmail.com Atefeh Khalili Atefeh Khalili Pediatric Pathology Research Center of Shahid Beheshti University of Medical Sciences, Tehran, Iran ati.ak61@gmail.com Reza Shiari Reza Shiari Department of Pediatric Rheumatology, Shahid Beheshti University of Medical Sciences, Tehran, Iran shiareza@yahoo.com Henoch-Schonlein purpura immunoglobulin A vasculitis children Factor 13 ## ##

Malignant Ovarian Steroid Cell Tumor: A Very Rare Tumor in Children 2 145 146 2020/05/272020/02/292020/04/72020/04/242020/02/272020/02/292020/03/202020/12/31 1399/10/11 2020/10/72020/11/22020/12/82020/11/22020/09/222020/09/262020/10/72020/12/11 1399/9/21 Fatemeh Malek Fatemeh Malek Pediatric Congenital Hematologic Disorders Research Center, Research Institute for Children’s Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran Atbin Latifi Atbin Latifi Pediatric Congenital Hematologic Disorders Research Center, Research Institute for Children’s Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran Saeed Habibi Saeed Habibi Pediatric Congenital Hematologic Disorders Research Center, Research Institute for Children’s Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran ## ##